Akron Children's Hospital and Dr. Blaise Congeni

I decided to repost a comment left on the facebook group page of Lemierre's Syndrome Fighters and Survivors because I have heard a lot of people searching for information on this disease and mentioned Akron hospital previously. If you are searching and discover the name Dr. Blaise Congeni, maybe it will help someone.

Here is the post: "TammyNgreg Clark Wow! Another akron Childrens Lemierres patient! So happy to hear your son is doing so well now! Congeni is AWESOME! He did an M&M conference on Lemierres using Stephanies case. She was the 19th case at children's when she was admitted and according to the dr's at that time she was the sickest Lemierres patient they had seen. We have taken Steph to childrens Er several times since because of her throat and I am so happy they know to take any visit very serious. I think awarness is spreading! I had introuduced Congeni to this group when he was gathering everything for the M&M conference. He was so happy to see there was such a group! The man is pasionate that is for sure! You could not be more right when u said life can change fast and all of us that have experience with Lemierres know what a wild scary ride it is! I am so happy that your son was able to be treated right away! Recovery can take time but as each day passes he will become stronger. I wish your son your family and you the best!"

Good update leads on Lemierres from group called Lemierre's Syndrome Fighters and Survivors

One mother, like myself, is attempting to correct inaccurate information reported in the news. Ironically, I worked for this newspaper group several years ago in Lake Tahoe. Mother Laurie Quarnberg posted in a group I am a member of Lemierre's Syndrome Fighters and Survivors several corrections in the article that appreared in the Greely Tribune in Colorado. I want to make sure her voice  is heard so inaccuracies are not replicated. 

Here is a portion of the story: 

When 16-year-old Madison Quarnberg of Greeley started showing symptoms of a sore throat, she didn’t know that it could almost cost her everything. Madison had symptoms of a sore throat and fever, so her mother, Laurie Quarnberg, took her to the hospital, where the doctor said that she had an infection. She was sent home with a round of antibiotics that was supposed to cure her. “I was really scared that first day,” Laurie said. “I knew something was wrong because she had a fever and wasn’t eating.” 

Please go to this link to see the full story. https://attachment.fbsbx.com/file_download.php?id=407422199369635&eid=ASvsxgBEbNeO5MWTMQoKLlO9tlyVRnlxZVyXdcOdxShGE_V6GmKHYc_vaC49xyFCKpk&inline=1&ext=1378332672&hash=AStyBv_CwX0ooWMK

Increase Awareness of Lemierre's Syndrome

Recently, I was thinking about the title of our blog: Bri's Hope for Cure of Lemierre's Syndrome. I think maybe what I really meant to call it and the reason why I started a blog, is because I wanted to increase AWARENESS of what Lemierre's is and how to diagnose it. The diagnosis is so important that maybe we should have called this blog: Increase Awareness of Lemierre's Syndrome. If treatment is begun, then the patient at least has a chance.

The problem is because it's The Forgotten Disease or The Mysterious Disease, treatment is delayed because of no diagnosis or wrong diagnosis and patients suffer or they die. My daughter was in excruciating pain for weeks. I couldn't help her so I felt helpless. But my helplessness was even deeper because not only could I help her with the pain, but her pain seemed to travel to different parts of her body. She complained of neck pain for weeks. Her body would go through fevers on and off. Then she was fine. Then it would reappear. It was so frustrating and scary.

While reading through Bri's medical records after we left the hospital, I found an excerpt from her main doctor who mentions it in her first record speculating what she may have. He later told me they had treated a young man several months before with the same symptoms. That was the reason he said he thought it possibly could be Lemierre's. What luck! This is a very rare disease with only approximately 160 reported cases in the last 100 years.

Lemierre's Syndrome: What is it?

I have learned a few things over the years about Lemierre's Syndrome: 

1. The medical term is REALLY hard to remember! ie., also known as postanginal sepsis and human necrobacillosis.

2. Lemierre's is called a syndrome or/and a disease. It is the  the occurrence of thrombophlebitis of the internal jugular vein (IJV) in the presence of an oropharyngeal infection.

3. It's commonly found in the mouth.

4. What are symptoms? Starts with sore throat, fever, weakness, neck swelling and progresses to shortness of breath, chest pain and possibly respiratory failure.

5. What are the characteristics? Septic blood clot formation in a jugular neck vein with emboli frequently traveling into the lungs and possibly joints and brain. 

6. What is treatment? Treatment includes antibiotics along with other measures such as ventilation, blood thinners, surgery and chest tubes. 

In Bri's case, she underwent two surgeries and then later when they found blood clots in her lungs and arms (yes, arms!) she began blood thinner therapy.

This disease was SO tough to diagnose because it appeared she had strept throat or something similar. Before she was diagnosed, I kept thinking, well, maybe she has one of those rare flus that strike people and there is no cure. In her case, the blood test was the first responder of saving her life! Because it found out she had sepsis (bacteria) in her blood. 

I hope these simple bullet points help someone searching for an answer to an unknown and undiagnosed case of Lemierre's Syndrome.

Monsters Inside Me on Animal Planet

I found the most recent story about a Lemierre's diagnosis while I was reading a blog I frequent about a young teen, Justin Rodgers, who fought Lemierre's and lost his battle. His sister has grown the nonprofit she established for him after his death. I have seen it grow over the years. Here is a link to his site: 
http://lemierresyndromejrodgers.blogspot.com/

Anyways, the story I read on her blog was about a story which aired in December last year on Animal Planet. Here is a brief synopsis which I am quoting from the Press Enterprise: 

"Abram, 23, was admitted to the hospital in April 2010 after complaining of a sore throat. He was subsequently diagnosed by Roberts and Dr. Tim Stevens, a former Arrowhead Regional oral surgery resident, with Lemierre’s — which is named after a French bacteriologist who, in 1936, documented 20 cases in which throat infections migrated to the bloodstream, potentially damaging vital organs. Abram was hospitalized for 28 days in the intensive care unit."

"An Arrowhead Regional Medical Center physician and a former patient who overcame a potentially deadly and rare disease known as Lemierre’s Syndrome will be featured in an upcoming episode of “Monsters Inside Me,” an Animal Planet Network television show."

Here is the link to the printed story: http://www.pe.com/local-news/san-bernardino-county/san-bernardino-county-headlines-index/20121205-colton-doctor-former-patient-to-be-on-tv.ece

I often think of Justin Rodgers because he was a healthy teen, like my daughter Bri, and he died and she is alive and thriving now at University California Santa Barbara. 

Primary Treatment: Antibiotics. Proper diagnosis is the biggest challenge!

Antibiotics are usually the primary treatment for Lemierre's. In my daughter's case, diagnosis came very late. We kept getting sent home with the possible diagnosis of "strept throat." 

Trying to find an underlying diagnosis for many conditions can be a very long and frustrating experience. With more rare conditions, like Lemierre's, a diagnosis can often take a long time. But the patient may not have the time to wait.

Lemierre's is considered a RARE disease. There are two other names for this disease:

2. Necrobacillosis and 

3. Oropharyngeal infection leading to secondary septic thrombophlebitis of the internal jugular vein

The important thing to remember is to keep pushing and be your own child's BEST advocate if doctor's cannot figure out or properly diagnose what's wrong. It's in cases like these you have to be the advocate and be involved. It's not the doctor's fault. 

In our case, I am grateful after a number of trips to the emergency room, one doctor at Scripps Encinitas Medical Center just happened to take a blood test. That is the instance they discovered my daughter Bri had sepsis in her blood. The clot in her jugular vein was spewing out bacteria into her blood. It is that one doctor who made the decision to take a blood test that initially saved her life!

Is Lemierre's cureable?

I found the following on netdoctor.com. I believe it simplifies what exactly Lemierre's is. Reprinted here from netdoctor.com

Question

What is Lemierre's disease?

Answer

Lemierre's syndrome is a rare complication of tonsillitis and other infections of the throat.

It was first recognised by a French physician in 1936 and is increasingly uncommon nowadays due to the use of effective antibiotics early on in the course of a bacterial illness.

In simple terms, Lemierre's syndrome describes an illness associated with the above and results in inflammation of the internal jugular vein.

This can lead to septicaemia (blood poisoning) and areas of infection at places elsewhere in the body.

The diagnosis is confirmed when the responsible bacteria can be grown from a sample of blood ('blood culture').

Treatment is by high dose antibiotics in hospital, usually straight in to a vein ('intravenously') and is continued for a period of up to six weeks after discharge from the hospital.

Because of the severity of the illness, and the susceptibility of the patient to develop septic areas within the lungs as a result of the original infection spreading.

Hospital admission is always needed and sometimes artificial ventilation for a while on an Intensive Care Unit (ICU).

With successful antibiotic treatment started promptly, a full recovery is to be expected, which, unfortunately, wasn't so often the case in Lemierre's day. Youngsters suffering from this complaint could easily succumb.

It sounds all in all like a particularly nasty infection and I for one am very glad that it is becoming a more rare occurrence these days.

Yours sincerely

The Medical Team

Read more: http://www.netdoctor.co.uk/ate/ent/203158.html#ixzz2ag8CuH80 
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Lemierre's Is Known As The Forgotten Disease

Lemierre's Syndrome: Forgiven, but Not Forgotten

there are several articles relating to Lemierre's in the Chest Journal Official College of Chest Physicians...

article reprinted here of a case of a 25 year old male.

Chest Infections | October 2012

Susan Mucha*, MD; Nicole Secca, MD; Kameron Ashker, MD; Marvin Balaan, MD; Tariq Cheema, MD

Author and Funding Information

Chest. 2012; 142(4_MeetingAbstracts):177A. doi:10.1378/chest.1390664

Text Size: A A A

Article

SESSION TYPE: Infectious Disease Case Report Posters II

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: Lemierre’s syndrome, or postanginal sepsis, begins with an oropharyngeal infection, progresses to septic thrombophlebitis of the internal jugular vein and septic metastasis. We present a young man with Lemierre’s syndrome complicated by respiratory failure.

CASE PRESENTATION: A 25-year-old healthy male presented to his PCP complaining of fevers, chills, sore throat, myalgias, and lymphadenopathy. Two days prior, he had kissed a woman with infectious mononucleosis. A work-up for EBV and streptococcal pharyngitis was negative. His symptoms progressed to nausea, emesis, cough, and weakness. While shopping a week later, EMS noted his profound weakness and brought him to his local hospital for dehydration. Blood cultures grew gram-negative rods later identified as Fusobacterium species. The patient was started on piperacillin-tazobactam and levofloxacin. A neck CT showed thrombosis involving the left internal jugular vein with extensive soft tissue changes along the left carotid sheath, contiguous with the left palatine tonsil. There was no abscess. A chest CT showed bilateral infiltrates suspicious for septic emboli. The patient was transferred to our MICU where he was emergently intubated for severe hypoxemia and worsening radiographic infiltrates. He had left neck tenderness and lymphadenopathy. The patient was treated with a regimen of piperacillin-tazobactam, clindamycin and intravenous heparin. He was extubated on ICU Day 7 and was discharged on Hospital Day 14 on amoxicillin-clavulanate for four more weeks and warfarin for three months.

DISCUSSION: This case describes Lemierre’s Syndrome that progressed to sepsis and respiratory failure. Andre Lemierre first published 20 cases in 1936, 18 patients died. The syndrome is a complication of an oropharyngeal infection, most commonly caused by Fusobacterium species. If left untreated, it can progress to internal jugular vein septic thrombophlebitis and septic pulmonary emboli. Prior to the advent of penicillin, mortality was approximately 90%. With the advent of penicillin, the incidence declined significantly to approximately 1 case per million per year, thus the syndrome became known as the “Forgotten Disease.” However, the incidence has increased over the last two decades. Mortality ranges from 5% - 15%. Treatment is directed at anaerobes, but should include coverage for potential co-infections. There are no controlled studies to support full anticoagulation, but is often recommended if response to antibiotics is poor. Abscess drainage or jugular vein ligation may be required.

CONCLUSIONS: Despite its rarity, Lemierre’s Syndrome should remain in the differential diagnosis in a young adult presenting with sore throat, fevers, and malaise.

1) Riordan T. Human infection with Fusobacterium necrophorum (Necrobacillosis) with a focus of Lemierre’s syndrome. Clin Microbiol Rev 2007; 20:622-59.

DISCLOSURE: The following authors have nothing to disclose: Susan Mucha, Nicole Secca, Kameron Ashker, Marvin Balaan, Tariq Cheema

No Product/Research Disclosure Information

Allegheny General Hospital, Pittsburgh, PA

Lemierre’s Syndrome: Rare, but Life Threatening—A Case Report with Streptococcus intermedius

This is an open access article which I have permission to replicate here with proper attribution. Every article I find proves my layman's theory about the history of our family blood disorder which I believe is related to  Lemierre's. Consistent in all of the Lemierre's cases are the returns to the ER with the same symptoms.

Case Presentation

A middle-aged woman presented to the emergency room (ER) with complaints of severe neck pain and occipital headaches for one week, which were not relieved with analgesics. She denied fevers, sore throat, cough, shortness of breath, or any trauma to the neck. Past medical history was significant for epilepsy and prior episodes of supraventricular tachycardia (SVT). She denied smoking or illicit drug use. She had a dental scraping of her left mandibular molars two weeks prior to gingivitis. Vital signs were stable in ER. Physical exam was positive for neck tenderness and minimal restriction of neck movements. Laboratory data revealed a white blood cell (WBC) of  (neutrophils 79%). Computerized tomography (CT) scan of the head and neck without contrast and lumbar puncture were done to rule out subarachnoid hemorrhage. The results did not reveal any abnormalities. Hence the patient was discharged on muscle relaxants and analgesics.

She returned to the ER in 5 days with high grade fevers, worsening neck pain and a headache. Temperature was 101°F, heart rate 162/min, respiratory rate 16/min, blood pressure 152/96 mmHg, and oxygen saturation of 92% on room air. Pharyngeal exam showed no erythema, swelling, or exudates. There was no evidence of otitis media or an active gingivitis either. There was no dental caries noted at that time, and there was no heat or cold intolerance. Percussion tenderness was not present. Neck examination showed restriction in range of movements, and a tender cord-like mass was palpable on the left side of neck. Cardiopulmonary examination revealed diffuse crackles in both lungs and no cardiac murmurs.

Laboratory data showed a WBC count of  (bands 44%) and an ESR of 98 mm/hr. Complete metabolic profile (CMP), including electrolytes, renal function (BUN, creatinine), and liver enzymes (LFTs) were all within normal range. EKG showed SVT. Chest X-ray showed small bilateral pleural effusions and bilateral pulmonary infiltrates without cavitations. CT scan of the head and neck with contrast demonstrated a thrombus in the left internal jugular vein (IJV) (Figure 1) extending to left sigmoid sinus (Figure 2) and bilaterally into the cavernous sinus (Figure 3). There was diffuse edema around the soft tissues of the neck. Preliminary blood cultures grew gram-positive cocci in chains. She was started empirically on intravenous Clindamycin and Vancomycin. Unfractionated heparin was also started due to the extensive clot burden. Workup for autoimmune and hypercoagulable diseases was unrevealing for any abnormality. Autoimmune workup included anti-nuclear anti-bodies (ANA), rheumatoid factor (RF), cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA), perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA), anti-Ro/SSA and anti-La/SSB antibodies. Hypercoagulable workup included prothrombin time (PT), partial thromboplastin time (PTT), international normalized ratio (INR), factor V Leiden mutation, prothrombin gene mutation, protein C and S as well as antithrombin levels, anti-cardiolipin antibodies and lupus anticoagulant.

In summary, we present a middle-aged female who presented with severe neck pain and occipital headaches for a week who did not have fevers or a sore throat on initial presentation but did provide a history of dental work performed two weeks ago. A noncontrasted CT of the head and neck failed to reveal any pathology on admission, and she was discharged from the ER whereby she very rapidly deteriorated over the next five days and then presented with worsening fevers, headaches, neck pain, and a leukocytosis of 34,000. Five blood cultures performed over a period of six days grew Streptococcus intermedius, and a CT head and neck with contrast showed thrombosis of the jugular vein, cavernous sinuses, and left sigmoid sinus. Patient survived and recovered after eight weeks of ampicillin-sulbactam.

Lemierre’s syndrome is a rare disease, typically caused by the microorganism Fusobacterium necrophorum. Tonsillitis is the most common primary infection (87.1%) followed by mastoiditis (2.7%) and odontogenic infections (1.8%) [11, 12]. This is typically followed by invasion of the pharyngeal lateral wall and thrombophlebitis of the internal jugular vein followed by high grade bacteremia and septic seeding of vital organs, most commonly the lungs. It is quite likely that our patient developed LS secondary to the gingival scraping that she underwent two weeks before her symptoms started. S.intermedius is a rare causative organism, and only 2 case reports of LS were found with this bacterium [8, 13]. Escalona et al. [8] reported a case with LS due to S. intermedius. This patient presented with extensive mandibular swelling due to an infected molar and fevers, along with an edematous floor of the mouth on physical exam. Chemlal et al. [13], reported a patient with LS, who had a recent pharyngitis presenting with fever and lower chest pain related to multiple pulmonary abscesses. Pharyngitis is a single most common presentation of LS as mentioned by Wright et al. [14]. In contrast, our patient’s mouth examination was totally benign, and she did not have a history of sore throat in the recent past. Our patient’s benign presentation and normal oropharyngeal examination might have delayed her diagnosis. It is tempting to speculate that differences in virulence properties between S. intermedius andF. necrophorum which is the usual pathogen responsible for LS may have contributed to an atypical presentation. However, the previous 2 reported cases of S. intermedius [8, 13] did present with oropharyngeal signs. Therefore, it is important to recognize that LS can present without any signs of pharyngitis or an active dental or ear infection and hence can be missed in the early phases of infection [3].

Diagnosis using CT scan of the head and neck with IV contrast is considered superior to a neck ultrasound as it is better in locating the anatomical extension of the thrombus [4, 5]. CT scan in the absence of contrast may be of limited utility (as was the case in our patient). Blood cultures should be sent on a patient with persistent severe pharyngitis and signs of sepsis and even in patients presenting with fevers and severe neck pain. Penicillin is the drug of choice, but due to recent penicillin-resistant strains of Fusobacterium, drugs like Clindamycin or beta lactam/beta-lactamase inhibitor are preferred [7, 14, 15]. Therapy should be started as soon as the syndrome is suspected and should be continued for at least 6 weeks [14–16]. Surgical drainage of abscess and IJV ligation may be indicated for patients who fail to respond to antibiotics, as was done in the preantibiotic era, though the ligature is not frequently done now [4, 8]. Routine use of anticoagulation is controversial as there are no randomized trials, and sepsis-related thrombocytopenia is often seen in these cases [14, 16]. Anticoagulation should strongly be considered, if there is clot propagation involving the cavernous sinus or if there are septic emboli [4, 5,7]. However anticoagulation can increase the risk of bleeding and expansion of hematoma.

4. Summary

Lemierre’s syndrome usually presents in childhood but may present atypically in middle-aged people, as in our patient. It can happen after pharyngitis, otitis media, odontogenic infections, or dental procedures [4]. The number of reported cases is increasing, due to the restricted use of antibiotics for sore throat and tonsillitis [5, 7]. High grade bacteremia with Streptococcus intermedius due to septic thrombosis, without any signs of an oral or pharyngeal infection at the time of presentation, is a unique feature of this case. Further, the ability of other oral flora to be causative agents of Lemierre’s syndrome is not as well established and recognized as it is with Fusobacterium necrophorum. This suggests that a benign mouth exam should not exclude the diagnosis of LS. In light of this, we recommend that LS should be considered in the differential diagnosis in patients presenting with persistent sore throat, mastoiditis, recent history of a dental procedure, and/or signs of active gingivitis, accompanied with neck pain and swelling. Blood cultures should be obtained and CT imaging of the neck with IV contrast should be performed. This, in turn, will enable timely diagnosis and improved outcome.

Shalini Gupta¹ and Shehzad S. Merchant²

¹Department of Internal Medicine, Oregon Health Science University, P.O. Box BTE 119, 3181 SW Sam Jackson Park Road, Portland, OR 97239, USA
²Infectious Diseases Division, Department of Medicine, Buffalo General Hospital, University at Buffalo, 100 High Street, B-8, Buffalo, NY 14203, USA

Received 15 August 2012; Accepted 1 October 2012

Academic Editor: Anthony W. Chow

Copyright © 2012 Shalini Gupta and Shehzad S. Merchant. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.2.